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GUILLAIN -BARRE SYNDROME
University of Versailles Saint-Quentin en YvelinesRaymond Poincaré Teaching Hospital
Garches - France
STATEMENTS
1. Guillain-Barré Syndrome (GBS) is the mostfrequent cause of acute peripheral paralysis.
2. GBS is secondary to an acute immune-mediatedpolyneuropathy
3. GBS can be differentiated in various clinical andelectrophysiological sub-types
4. Its gravity includes respiratory failure, cardio-vascular autonomic dysfunction, and long-termdisability
INCIDENCE DU SYNDROME DE GUILLAIN -BARRE
1. INCIDENCE: 0.5–2.0 cases/100 000/year2. SEXRATIO (F/M): < 1.03. AGE RATIO (O/Y): > 1.04. OUTBREAK: NO SEASONAL VARIATION5. PRECEDING SYMPTOMS: 70-90%
DEFINITIONREQUIRED CRITERIA
1. Progressive weakness > 2 limbs2. Areflexia3. Progressionfor < 4 weeks4. Other cause of acute neuropathy excluded
SUPPORTIVE CRITERIA1. Mild sensory signs2. RaisedCSFprotein3. conductionblock in EMG
Van Doorn et al – Press Med - 2013
CRITERES DIAGNOSTIQUES
EVOLUTION
Van Doorn et al – Press Med - 2013
Van Doorn et al – Press Med - 2013
DIAGNOSTICS DIFFERENTIELS
Pas de syndrome confusionnelPas de syndrome inflammatoireps d’hypercellulorachie
GUILLAIN -BARRE SYNDROME COURSE
Day 0 Time
Motor deficitExtension Recovery
Plateau
SUBTYPES
1. Acute inflammatory demyelinatingpolyneuropathy (AIDP)
2. Acute motor axonal neuropathy (AMAN)3. Acute motor and sensory axonal
neuropathy (AMSAM)4. Miller Fisher syndrome (MFS)
PATHOPHYSIOLOGY
Hughes and Cornblath - Lancet - 2005
ANTIGANGLIOSIDE ANTIBODIES
Hughes and Cornblath - Lancet - 2005
ADMISSION1. ASCENDANT WEAKNESS (70%)
– Flaccid symetric areflexic weakness– Pure motor deficit: 18%
2. SENSORY SYMPTOMS (50 à 80 %)– Paresthesia, pain– Superficial and deep sensory impairment
3. CRANIAL NERVES– VII: 24-55%; IX -X: 6-46%; III-IV -VI: 5-13%– XII: 1 - 13%
4. DYSURIA (10 - 30%)
SCALES
MRC sum score
Kleyweg et al. - Muscle Nerve - 1991
Disability grade
SEVERITY AT ADMISSION
1. SWALLOWING IMPAIRMENT- In 6 to 46%
2. RESPIRATORY DYSFUNCTION- Respiratory symptoms in 40 to 60%- Vital capacity < 1 L in 16%
3. CV AUTONOMIC DYSFUNCTION- in about 15%- Correlated with weakness
ANTICIPATING ACUTE RESPIRATORY FAILURE
IN GUILLAIN -BARRE SYNDROME
MECHANICAL VENTILATION
1. Required in 20 to 30%2. Median time from admission to MV: 2 days3. Median MV duration: 21 days4. Up to 1/3 weaned from MV within 3 weeks
MECHANISMS
Diaphragm
Accessory
Abdominal
↓↓↓↓ CV
↓↓↓↓ Pimax
↓↓↓↓ Pemax
↓↓↓↓ cough
Atelectasia
Bulbar dysfunction
Aspiration
Phrenic
IX -X
Hypoventilation
Hypoxaemia
T8-T12
AdmissionOnset MV
EARLY PREDICTORS
EARLY CRITERIAE
LATE CRITERIAE
LATE PREDICTORS
Respiratory arrest
ICU ward
MV
No VM
CRITERIA FOR INTUBATION
MAJOR CRITERIA1. Signs of respiratory distress2. VC< 15 ml/kg, Pimax ou Pemax < 25 cm H2O3. PaCO2 > 6,4 kPa4. PaO2 < 7.5 kPa (FiO2= 0,21)
MINOR CRITERIA1. Expectoration inefficient 2. Severe bulbar dysfunction3. Atelectasis
Ropper Neurology 1985 - Wijdicks Neurology 1998
THE RISKS
• RESPIRATORY AND CARDIAC ARREST• ASPIRATION• ARDS
Orlikowski et al - ICM - 2007
N= 87VAP = 67 (75%)
BLOOD GASES «FAUX-AMIS »
1. Presence of hypercapnia or hypoxemia indicatesa severe respiratory muscle weakness andmisgives an impeding respiratory arrest
2. Conversely, absence of blood gases abnormalitydoes not rule out severe respiratory muscleweakness
HYPOXEMIA
• Can be inducedby increasedPaCO2– physiological law– Check that decrease in PAO2 is explained by
increase in PaO2
• Pneumoniae, atelectasia…– Check the chest x Ray
• Pulmonary embolism– To be suspected if chest x ray is normal– Frequent in this patient
EARLY PREDICTORS
INABILITY OR 95% CI p
PROGRESSION < 7 days 2.51 (1.7 – 3.8) < 0.0001
STAND 2.53 (1.4 – 3.3) < 0.0005
RISE ELBOW 2.99 (1.8 – 4.9) < 0.0001
RISE HEAD 4.34 (2.7 – 6.7) < 0.0001
COUGH 9.09 (4.0 – 20.00) < 0.0001
CYTOLYSIS 2.09 (1.4 – 3.2) < 0.0005
n = 722
Sharshar et al-Crit Care Med-2003
EARLY PREDICTORS
n= 196
Inability OR 95% CI p Value
PROGRESSION < 7 days
5.0 (1.4 – 5.7) < 0.003
HEAD 5.0 (1.9 – 12.5) < 0.0011
VC < 60% 2.86 (2.4 – 10.0) < 0.0001
Sharshar et al-Crit Care Med-2003
CHANGE IN VC WITH TIME
Durand et al – Neurology - 2005
MV
No MV
REPEAT THE MEASUREMENT OF VITAL CAPACITY
OTHER PREDICTORS
1. 30 to 50% fall in VC or VC < 20 ml/kg
2. 30% fall in Pimax or Pemax
or Pimax ou Pemax< 30 to 40 cmH2O 3. Bulbar dysfunction
Hahn et al Arch Neurol 2001
Chevrolet et al Am Rev Respir Dis 1991
OTHER PREDICTORS
Walgaard et al – Ann Neurol - 2010
ELECTROPHYSIOLOGY• Phrenic ENMG
– Not helpful (Durand – Neurology – 2005)• Standard ENMG
– Demyeliting form at higher risk (Durand–Eur J Neurol 2003)
– No risk if no conduction block on commonperoneal nerve + VC >80% (Durand –Lancet Neurology – 2006)
Durand et al – Lancet Neurology - 2006
CORTISOLEMIA
Variableno MV
(Group 2)MV > 24h(Group 3)
P*
n 60 17
[Cortisol]T0 (ng/ml) 20.4 ± 9.6 28.5 ± 12.1 0.01
[Cortisol]T60 (ng/ml) 42.4 ± 14.8 53.1 ± 16.8 0.05
Strauss et al – CCM - 2009
[Cortisol]T0 correlated with occurrence of dysautonomia
FEARN (%)
Marcadet et al - Submitted
0
20
40
60
80
100
Dyspnea Pain Weakness Uncertainty
No MVMV
AdmissionOnset MV
EARLY PREDICTORS
Inability to lift head
VC < 60%
+/- High cortisolemia
CPN + VC > 80%
EARLY CRITERIAE
LATE CRITERIAE
LATE PREDICTORS
Fall in VC
Swallowing dysfunction
Respiratory arrest
ICU ward
MV
No VM
VERY EARLY PREDICTORS
Onset-Admission < 7 days
Uncertainty
ADMISSION IN ICU
1. Progression less than 7 days2. Inability to lift head above the bed3. Bulbar dysfunction4. VC less than 60% or 20 ml/Kg
Pimax ou Pemax< 30 to 40 cmH2O5. VC fell by 30%
Pimax or Pemax fell by 30%6. CV autonomic dysfunction
The absence of these criteria does not exempt to assessregularly weakness, bulbar function and respiratoryfunction
TRACHEOSTOMY
Prolonged MV > 21 days1. Elder patients2. Preexisting pulmonary disease3. Pulmonary function dint/d12 ratio < 1
1. PF score = CV + Pimax + Pemax2. Sensitivity = 70%, specificity: 100%
Lawn and Wijdicks Muscle Nerve 1999, 2000
OTHER ABNORMALITIES
HYPONATREMIA E1. Hyponatremia < 133 mmol/L : 31%2. Pseudohyponatremia due to IvIg: 46%3. Hyponatremia: worst outcome ?
LIVER DYSFUNCTION
1. Cytolysis: 25%
2. Secondary to CMV: 25%
3. Predictors of MV
Colls Int Med J 2003; Oomes et al Neurology 1996;
Sharshar et al Crit Care Med 2003
TREATMENT
1. Specific treatment1. Plasma exchange (PE) NO IF INFECTION2. High-dose intravenous immunoglobulin (IvIg)
2. Supportive therapy1. Thrombosis prophylaxis2. Pain relief3. Psychological support4. CV support: atropine …5. Bladder catheter, nutrition, physiotherapy…6. Intercurrent diseases7. Early non-invasive mechanical ventilation
PLASMA EXCHANGE
Plasma exchange vs no treatment: DG at 4 weeks
Hughes et al - Brain - 2007
1. Benefit irrespective of severity2. Tested against placebo3. Tested up to 4 weeks after GBS onset
IMMUNOGLOBULIN
Hughes et al - Brain - 2007
Plasma exchange vs IvIg: DG at 4 weeks
1. Tested against PE
2. Tested in mild or severe GBS
3. Tested within 2 weeks after GBS onset
CORTICOSTEROID
Hughes et al - Brain - 2007
Corticosteroid vs placebo or no treatment: DG at 4 weeks
CONTRAINDICATIONS
PE
1. Infection
2. Hypotension
3. Haemorrhage
4. Clotting deficiency
IvIg1. IgA deficiency2. Allergy3. Renal failure
ALGORITHMS
Disability
Stage
Walking
(DG < 4)
Bedbound (DG =4)
MV (DG = 5)
Initial * 2 PE 4 PE or IvIg**
Deterioration 2 additional PE No additional treatment
Relapse No treatment or do initial treatment
No treatment or do initial treatment
*As soon as the diagnosis is confirmed** 0.4g/kg/ daily for 5 days
EVOLUTION
Van Doorn et al – Press Med - 2013
Raymond Poincaré
THANK YOU
MORBIDITYRESPIRATORY1. Tracheobronchitis 33 (29%)2. Pneumonia 27 (24%)3. Pneumothorax 6 (4%)
INFECTION1. Bacteremia 21 (15%)2. Catheter 3 (3%)3. Urinary tract 75 (66%)
THROMBOSIS1. Deep venous thrombosis 5 (4%)2. Pulmonary embolism 3 (3%)
OTHERS1. Hyponatremia (< 130 mmol/L) 28 (25%)
Henderson et al Neurology 2003
n = 114
RISK FACTORS OF MORBIDITY
Characteristic OR 95% CI p Value
Background illness 2.8 (0.8 - 9.1) 0.10
Plasma exchange 3.4 (1.1 – 10.5) 0.03
Prolonged MV 12.4 (3.7 – 41.3) < 0.0001
Henderson et al Neurology 2003
n = 114
ADMISSION IN ICU
1. Progression less than 7 days2. Inability to lift head above the bed3. Bulbar dysfunction4. VC less than 60% or 20 ml/Kg
Pimax ou Pemax< 30 to 40 cmH2O5. VC fell by 30%
Pimax or Pemax fell by 30%6. CV autonomic dysfunction
The absence of these criteria does not exempt toassess regularly weakness, bulbar function andrespiratory function
PUZZLEPathogen agent
1. C. jejuni
2. CMV
3. Others
Anti-Ganliosides
1. Ant-GM1
2. Anti-GM 2
3. Anti-G1QB
Subtypes
1. AIDP
2. AMAN
3. AMSAM
4. MFS
Clinic
1. Sensorimotor
2. Pure motor
3. Cranial nerves
Severity
1. Aspiration
2. Intubation
3. Dysautonomia
Outcome
1. Recovery
2. Sensory sequellae
3. Motor sequellae
WHY NORMOCAPNIC IS NOT « SAFE»
Tid
al V
olum
e
Normocapnic but no reserve because low
VC
Respiratory arrest if anything happpen
Healthy subjectCan increase his VT
Patients with NMD cannot
increase his VT
ACUTE FLACCID WEAKNESS
SENSORIMOTOR PARALYSIS
PURE MOTOR PARALYSIS
1. ± MRI
2. CSF ANALYSIS
3. BLOOD TESTS (ESR)
4. EMG (Axonal PN, Demyelinating PN)
1. BLOOD TESTS (K+)
2. CSF ANALYSIS
3. EMG (Myopathy, NMJ, Neuropathy)
Pyramidal signsSensory level
Cauda-equina syndrome
SPINAL CORD MRI
ACUTE SENSORIMOTOR HYPOREFLEXIC PARALYSIS
ACUTE SENSORIMOTOR HYPOREFLEXIC PARALYSIS
ESR NORMAL ESR INCREASED
CSFNORMAL
TOXIC (Thallium, arsenic…)METABOLIC (Gly, Vit …)VASCULITIS (SLE…)PRIMARY GBS
VASCULITIS (SLE…)
INCREASEDCSF CELLS
MENINGORADICULITIS MENINGORADICULITIS
INCREASEDCSF
PROTEIN
PRIMARY GBSCANCER, LYMPHOMA,VASCULITIS, DIPHTERIA,HIV.
CANCER, LYMPHOMA,VASCULITIS, DIPHTERIA,HIV.
PURE MOTOR HYPOREFLEXIC DEFICIT
SIGNS K+ CSF EMG
PERIODIC PARALYSIS
EXERCISE ↑↓↑↓↑↓↑↓ ↔↔↔↔ MYOPATHY
MYASTHENIA GRAVIS
VARIATIONEYE MVT
↔↔↔↔ ↔↔↔↔ NM JUNCTION
BOTULISMFOOD
POISONNINGPUPILL
↔↔↔↔↔↔↔↔ NM JUNCTION
POLIOMYELITIS
TRAVELDIARRHEA
↔↔↔↔↑↑↑↑ CELLS
ANTERIOR HORN CELLS
PORPHYRICNEUROPATHY
CONFUSIONPAIN
↔↔↔↔↔↔↔↔ POLY
NEUROPATHY
PRIMARYGBS
INFECTIONASCENDANT
↔↔↔↔↑↑↑↑ PROTEIN
POLY NEUROPATHY